Original article
Pediatric cardiac
Use of Oral Budesonide in the Management of Protein-Losing Enteropathy After the Fontan Operation

https://doi.org/10.1016/j.athoracsur.2009.09.063Get rights and content

Background

Intestinal inflammation is a component of the pathophysiology of protein-losing enteropathy after the Fontan operation. Oral controlled-release budesonide is 90% metabolized at first pass through the liver, has high enteric anti-inflammatory activity and relatively low systemic effects, and may be an ideal agent for use in treating this disease.

Methods

Budesonide was administered to 9 patients (4 male) with protein-losing enteropathy after the Fontan operation. The median interval between the Fontan operation and diagnosis of protein-losing enteropathy was 4 years (range, 0.1 to 13.3). Prior interventional therapy included pulmonary artery stent (1), fenestration (3), pacemaker placement (3) and Fontan revision (2). Prior medical therapy included oral prednisone (5), heparin (4), sildenafil (2), infliximab (1), and octreotide (1), all without persistent success. The starting daily dose of budesonide was 9 mg for patients 4 years old or older, and 6 mg for patients less than 4 years of age.

Results

Mean serum albumin level 3 months before starting budesonide was 1.9 g/dL (range, 1 to 2.4 g/dL). Serum albumin level improved in all patients within 6 months of starting budesonide (mean 2.9 g/dL; range, 2.2 to 3.8 g/dL). Albumin levels of 3 g/dL or more were achieved in 8 of 9 patients within a median of 4.3 months (range, 2 to 25). Side effects included Cushingoid features and osteoporosis (3), infection requiring antibiotic treatment (5), and acne exacerbation (1). Weaning from high initial dose to a lower dose was possible with sustained effect; however, discontinuation of budesonide resulted in recurrence of hypoalbuminemia.

Conclusions

Oral budesonide is an effective therapy for treating protein-losing enteropathy after the Fontan operation. To maintain response, low-dose therapy must be continued.

Section snippets

Patient Population

Between January 2005 and May 2009, 9 patients who had the Fontan operation and active PLE were started on a regimen of oral CR-budesonide. Patient characteristics including cardiac findings on echocardiography and cardiac catheterization were recorded. Chart review was performed with recording of response to oral CR-budesonide, efficacy, and side effects. Diagnosis of PLE was based on the clinical findings of peripheral edema or ascites and confirmation of hypoalbuminemia on multiple samples

Patient Characteristics

Median age at performance of the Fontan operation was 2.7 years (range, 1.2 to 10.8). By nature of their congenital heart disease, the functioning systemic single ventricle was a left ventricle in 4 patients and a right ventricle in the other 5. Median duration between the Fontan operation and initial diagnosis of PLE was 4 years (range, 0.1 to 13.3). Types of Fontan operation included lateral tunnel (n = 6), atriopulmonary (n = 2), and extracardiac (n = 1). Four were fenestrated at initial

Comment

To date, our understanding of PLE after the Fontan operation remains limited; however, we have developed a conceptual framework that is proving effective for management. We postulate that PLE after the Fontan operation is based on a fundamental alteration in hemodynamics, inherent in the physiology of relatively low cardiac output and elevated systemic venous pressure, findings present to some degree in all subjects with a Fontan circulation. The combination of low cardiac output and elevated

References (34)

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    Citation Excerpt :

    The symptoms of PB can be mitigated by using aerosolized tissue plasminogen activator which may break down cast material facilitating their expectoration; the use of inhaled bronchodilatator, mucolytics and steroid has been reported also [68]. Butesonide is reported to reduce the inflammatory status of the gut meliorating the loss of proteins [69]. Common therapeutic strategies are reducing the systemic venous pressure with aggressive pulmonary vasodilatator therapy, such as sildenafil or bosentan, treating the Fontan sequelae as the stenosis of the Fontan pathway and/or creating a fenestration (Fig. 4).

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